Korean Journal of Nephrology 2008;27(2):243-247.
A Case of Splenectomy in a Patient with Refractory Thrombotic Thrombocytopenic Purpura
Jae-Won Lee, M.D.1, Sun-Chul Kim, M.D.1, Se-Won Oh, M.D.1, Jin-Joo Cha, M.D.1, Hye-Won Kim, M.D.1, Chang-Su Boo, M.D.1, Ji-Eun Lee, M.D.2, Young-Joo Kwon, M.D.1 and Heui-Jung Pyo, M.D.1
Department of Internal Medicine1
College of Medicine, Korea University, Seoul; Department of Internal Medicine2
Wonkwang University School of Medicine, Gunpo, Korea
증례 : 비장적출술로 호전된 불응성 혈전성 혈소판감소성 자반증 1예
이재원1, 김선철1, 오세원1, 차진주1, 김혜원1, 부창수1, 이지은2, 권영주1, 표희정1
고려대학교 의과대학 내과학교실1, 원광대학교 의과대학 내과학교실2
Abstract
The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP) and the survival rate was increased from 10 to 80-90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic challenge. We describe here a patient who partially responded to plasma exchange therapy, but remained dependent on plasma infusions. To discontinue plasma therapy, several attempts using agents such as rituximab, vincristine, and cyclosporine A had been tried, but all failed. After splenectomy, serum LDH and blood platelet count were normalized. Plasmapheresis were we able to discontinue after 2 weeks of splenectomy. Steroid and cyclosporine were tapered off after 3 months and 5 months after splenectomy respectively, and the patient has been staying in remission ever since. We suggest that splenectomy is a worthwhile treatment option in patients with refractory TTP. Key Words : Thrombotic thrombocytopenic purpura, Splenectomy


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